The mechanistic GEMMs of oncogenic histones

Anders M. Lindroth, Yoon Jung Park, Verónica Matía, Massimo Squatrito

Research output: Contribution to journalReview articlepeer-review

1 Scopus citations


The last decade's progress unraveling the mutational landscape of all age groups of cancer has uncovered mutations in histones as vital contributors of tumorigenesis. Here we review three new aspects of oncogenic histones: first, the identification of additional histone mutations potentially contributing to cancer formation; second, tumors expressing histone mutations to study the crosstalk of post-translational modifications, and; third, development of sophisticated biological model systems to reproduce tumorigenesis. At the outset, we recapitulate the firstly discovered histone mutations in pediatric and adolescent tumors of the brain and bone, which still remain the most pronounced histone alterations in cancer. We branch out to discuss the ramifications of histone mutations, including novel ones, that stem from altered protein-protein interactions of cognate histone modifiers as well as the stability of the nucleosome. We close by discussing animal models of oncogenic histones that reproduce tumor formation molecularly and morphologically and the prospect of utilizing them for drug testing, leading to efficient treatment and cure of deadly cancers with histone mutations.

Original languageEnglish
Pages (from-to)R226-R235
JournalHuman Molecular Genetics
StatePublished - 2021

Bibliographical note

Funding Information:
We would like to thank Dr Oren Becher for providing the RCAS-H3.3K7M vector. This work was supported by the National Research Foundation (NRF) of the South Korean government [2017R1E1A1A01073670 to A.M.L.]; the National Cancer Center Korea [1810050-1 to A.M.L.], and the Seve Ballesteros Foundation [to M.S.] Conflict of Interest statement. The authors declare no competing interests.

Publisher Copyright:
© The Author(s) 2020. Published by Oxford University Press. All rights reserved.


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